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1 Cards in this Set
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1. What is Engelman dysplasia?
2. How do you differentiate from osteopetrosis? |
1.
- Autosomal Dominant diaphyseal dysplasia - Characterized by IRREGULAR and INHOMOGENEOUS thickening and sclerosis of DIAPHYSEAL CORTICES leading to expansion of the shaft with narrowing of the medullary cavities (mimics melorheostosis) - Medullary cavities may be compromised to the degree that extramedullary hematopoeisis develops. - The epiphyses and metaphyses are characteristically spared. - Skull base sclerosis is common and significant as it often leads to cranial nerve encroachment. - Involvement is largely symmetric (On bone scan, it mimics HOA) - **Sclerotic posterior vertebra and neural arches + hepatosplenomegaly 2. Engelmann's disease affects only the diaphysis of a tubular bone, whereas osteopetrosis involves the entire bone. Engelmann's disease rarely affects the bones of the hands and feet, clavicles, pelvis, ribs, and scapula. |
